title: Relevance of dietary iron intake and bioavailability in the management of HFE hemochromatosis: a systematic review.
abstract: BACKGROUND
  Hereditary hemochromatosis (HH) leads to iron loading because of a disturbance in the negative-feedback mechanism between dietary iron absorption and iron status. The management of HH is achieved by repeated phlebotomies.
  
  
  OBJECTIVE
  We investigated whether HH patients would benefit from a diet with low iron intake and bioavailability.
  
  
  METHODS
  We performed a systematic review of studies that linked iron bioavailability and status with dietary factors in subjects with diagnosed HH. Studies on heterozygotes for the HFE mutation were excluded.
  
  
  RESULTS
  No prospective, randomized study was reported. Nine studies that directly measured iron bioavailability from test meals in HH patients have been described as well as 3 small, prospective, longitudinal studies in HH patients. Eight cross-sectional studies were identified that investigated the effect of dietary composition on iron status. Calculations of iron bioavailability in HH were made by extrapolating data on hepcidin concentrations and their association with iron bioavailability. The potential reduction in the yearly amount of blood to be phlebotomized when restricting dietary iron absorbed was estimated in the 3 longitudinal studies and ranged between 0.5 and 1.5 L. This amount would be dependent on individual disease penetrance as well as the dietary intervention.
  
  
  CONCLUSIONS
  Despite the limited quantitative evidence and the lack of randomized, prospective trials, dietary interventions that modify iron intake and bioavailability may affect iron accumulation in HH patients. Although this measure may be welcome in patients willing to contribute to their disease management, limited data exist on the clinical and quality-of-life benefit.
authors.affiliation: Division of Human Nutrition and Science Shop, Wageningen University, Wageningen, Netherlands.
authors.forename: Diego
authors.initials: D
authors.lastname: Moretti
authors.forename: Gerrigje M
authors.initials: GM
authors.lastname: van Doorn
authors.forename: Dorine W
authors.initials: DW
authors.lastname: Swinkels
authors.forename: Alida
authors.initials: A
authors.lastname: Melse-Boonstra
mesh_terms: D000042:Absorption; D023181:Antimicrobial Cationic Peptides / Q000097:blood; D001682:Biological Availability; D004032:Diet; D006432:Hemochromatosis / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D064451:Hepcidins; D006579:Heterozygote; D006801:Humans; D019266:Iron, Dietary / Q000008:administration & dosage* / Q000493:pharmacokinetics*; D009154:Mutation; D019683:Penetrance
chemical_list: D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D019266:Iron, Dietary